Outcomes of cochlear implantation in Usher syndrome: a systematic review.

PURPOSE: This study is a systematic review of the literature which seeks to evaluate auditory and quality of life (QOL) outcomes of cochlear implantation in patients with Usher syndrome. METHODS: Systematic review of studies indexed in Medline via PubMed, Ovid EMBASE, Web of Science, CENTRAL and clinicaltrials.gov was performed up to March 9th 2022, conducted in accordance with the PRISMA statement. Patient demographics, comorbidity, details of cochlear implantation, auditory, and QOL outcomes were extracted and summarized. RESULTS: 33 studies reported over 217 cochlear implants in 187 patients with Usher syndrome, comprising subtypes 1 (56 patients), 2 (9 patients), 3 (23 patients), and not specified (99 patients). Auditory outcomes included improved sound detection, speech perception, and speech intelligibility. QOL outcomes were reported for 75 patients, with benefit reported in the majority. CONCLUSIONS: Many patients with Usher syndrome develop improved auditory outcomes after cochlear implantation with early implantation being an important factor.

Throat and voice problems in Ehlers-Danlos syndromes and hypermobility spectrum disorders.

A small number of case reports and observational studies describe chronic nasal congestion, upper airway obstruction, dysphonia, vocal cord abnormalities, and swallowing abnormalities in the Ehlers-Danlos syndromes. Little is known of the causes and therefore treatments of these, yet they are not uncommon findings in persons with hypermobility-related conditions presenting in the healthcare setting. We have a specialist multidisciplinary ear, nose, and throat and speech therapy practice with accumulating observational and empirical experience of managing these conditions, which include altered voice, choking, high dysphagia and anterior and deep neck pains. Here, we present our experience, some illustrative cases, and suggestions for future work in this evolving field.

Top 100 cited manuscripts in aortic valve replacement: A bibliometric analysis.

This bibliometric analysis aims to identify publications and highlight the key areas that have shaped modern clinical practices for aortic valve replacement (AVR). In this paper, the top 100 most cited manuscripts for AVR are analyzed. The Thomson Reuters Web of Science database was searched using the terms "aortic valve replacement," "AVR," "sAVR," "tAVR," or "TAVI." The results were ranked by citation number and the top 100 articles were further analyzed by evaluating the subject, author, journal, year of publication, institution, and country of origin. Thirty-thousand and eight hundred eligible papers were examined, with an accumulation of 81 851 citations in total and a mean citation of 819 per manuscript (ranged: 344-4180). The New England Journal of Medicine had the most manuscripts whereas Circulation had the most citations. The number of citations has also significantly increased for articles published after 2000.The most cited manuscript highlighting the management of valvular heart disease, was written by Baumgartner et al By providing the most influential references, this work serves as a comprehensive guide to topics of interest in the field of AVR.

Diagnosis and management of leptomeningeal disease secondary to grade IV astrocytic glioma.

A man in his mid-40s presented to hospital with confusion, headache and feeling generally unwell. He had had a total resection of a grade IV astrocytic glioma 1 year prior. Initial observations, blood tests and CT head scan were unremarkable for acute features to explain the patient's presentation. However, an MRI head scan on this admission demonstrated a clear communicating hydrocephalus with new abnormal leptomeningeal enhancement, consistent with leptomeningeal metastatic infiltration by glioma. Lumbar puncture cytology and biochemistry supported this interpretation. As a small district general hospital in rural Wales, we discuss the experience of diagnosis and coordination of specialist input from a multidisciplinary team. We share the challenges of managing leptomeningeal disease in the COVID-19 pandemic, in the context of the additional risks this presents with chemotherapy-induced immunosuppression.

Cochlear Implant Outcomes in Patients With Otosclerosis: A Systematic Review.

OBJECTIVE: This study aimed to establish hearing outcomes after cochlear implantation in patients with otosclerosis. MATERIALS AND METHODS: We conducted a systematic review and narrative synthesis. Databases searched were as follows: MEDLINE, PubMed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov . No limits were placed on language or year of publication. The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. RESULTS: Searches identified 474 abstracts and 180 full texts, with 68 studies meeting the inclusion criteria and reporting outcomes in a minimum of 481 patients with at least 516 implants. Patient-reported outcome measures (PROMs) were reported in five studies involving 51 patients. Intraoperative adverse events/surgical approach details and preoperative radiological assessment were reported in 46 and 38 studies, respectively. The methodological quality of included studies was modest, predominantly consisting of case reports and noncontrolled case series with small numbers of patients. Most studies were Oxford Centre for Evidence Based Medicine grade IV. DISCUSSION: Access to good rehabilitation support is essential to achieving the good hearing outcomes and PROMs that can be expected by 12 months after implantation in most cases. There was a significant association between the radiological severity of otosclerosis and an increase in surgical and postoperative complications. Postoperative facial nerve stimulation can occur and may require deactivation of electrodes and subsequent hearing detriment. CONCLUSIONS: Hearing outcomes are typically good, but patients should be counseled on associated surgical complications that may compromise hearing. Modern diagnostic techniques may help to identify potentially difficult cases to aid operative planning and patient counseling. Further work is needed to characterize PROMs in this population.